{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,10]],"date-time":"2026-11-10T22:10:50Z","timestamp":1794348650454,"version":"4.9.0"},"reference-count":17,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2022,5,28]],"date-time":"2022-05-28T00:00:00Z","timestamp":1653696000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,5,28]],"date-time":"2022-05-28T00:00:00Z","timestamp":1653696000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Respir Res"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Pirfenidone slows down disease progression in idiopathic pulmonary fibrosis (IPF). Recent studies suggest a treatment effect in progressive pulmonary fibrosis other than IPF. However, the safety and effectiveness of pirfenidone in asbestosis patients remain unclear. In this study, we aimed to investigate the safety, tolerability and efficacy of pirfenidone in asbestosis patients with a progressive phenotype.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>This was a multicenter prospective study in asbestosis patients with progressive lung function decline. After a 12-week observational period, patients were treated with pirfenidone 801\u00a0mg three times a day. Symptoms and adverse events were evaluated weekly and patients completed online patient-reported outcomes measures. At baseline, start of therapy, 12 and 24\u00a0weeks, in hospital measurement of lung function and a 6\u00a0min walking test were performed. Additionally, patients performed daily home spirometry measurements.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>In total, 10 patients were included of whom 6 patients (66.7%) experienced any adverse events during the study period. Most frequently reported adverse events were fatigue, rash, anorexia and cough, which mostly occurred intermittently and were reported as not very bothersome. No significant changes in hospital pulmonary function (forced vital capacity (FVC), diffusion capacity of the lung for carbon monoxide (DLCO), 6\u00a0min walking test or patient-reported outcomes measures before and after start of pirfenidone were found. Home spirometry demonstrated a FVC decline in 12\u00a0weeks before start of pirfenidone, while FVC did not decline during the 24\u00a0week treatment phase, but this difference was not statistically significant.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Treatment with pirfenidone in asbestosis has an acceptable safety and tolerability profile and home spirometry data suggest this antifibrotic treatment might attenuate FVC decline in progressive asbestosis.<\/jats:p>\n                <jats:p><jats:italic>Trial registration<\/jats:italic> MEC-2018-1392; EudraCT number: 2018-001781-41<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12931-022-02061-2","type":"journal-article","created":{"date-parts":[[2022,5,28]],"date-time":"2022-05-28T13:05:43Z","timestamp":1653743143000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Safety and tolerability of pirfenidone in asbestosis: a prospective multicenter study"],"prefix":"10.1186","volume":"23","author":[{"given":"Jelle R.","family":"Miedema","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catharina C.","family":"Moor","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcel","family":"Veltkamp","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Baart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natascha S. 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All patients gave written informed consent to participate.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"JM reports consulting fees from Boehringer Ingelheim; lecture fees from Boehringer Ingelheim, F. Hoffmann-La Roche, Novartis, outside the submitted work.\u00a0CM reports grants and fees from Boehringer-Ingelheim and Hoffmann-la Roche, outside the submitted work. All grants and fees were paid to the institution.\u00a0MV reports consulting fees and lecture honoraria from Boehringer Ingelheim; lecture honoraria from Chiesi; outside the submitted work.\u00a0MW reports grants from Boehringer Ingelheim, F. Hoffmann-La Roche consulting fees from Boehringer Ingelheim, F. Hoffmann-La Roche, Galapagos, Bristol Myers Squibb, Galecto and Respivant; lecture fees from Boehringer Ingelheim, F. Hoffmann-La Roche, Novartis; travel support from Boehringer Ingelheim, F. Hoffmann-La Roche; and participation in data safety monitoring boards for Savara and Galapagos. All grants and fees were paid to the institution.\u00a0RM reports consulting fees and lecture honoraria from Boehringer Ingelheim; consulting honoraria from Galapagos; outside the submitted work.\u00a0All other authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"139"}}